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Staphylococcus aureus is a troublesome pathogen, responsible for a broad range of clinical manifestations, ranging from benign skin infections to life-threatening conditions such as endocarditis and osteomyelitis. The kidney can be affected through a rapidly progressive glomerulonephritis mediated by an inflammatory reaction against a superantigen deposited in the glomerulus during the infection’s course. This glomerulopathy has a poor prognosis, often leading to chronically impaired kidney function, eventually progressing to end-stage renal disease. Treatment rests on antibiotherapy. Despite the inflammatory role in this disease’s pathophysiology, most authors discourage a simultaneous immunosuppressive approach given the concomitant infection. However, there are some reports of success after administration of systemic corticosteroids in these patients. We present a 66-year-old man with a staphylococcus-induced glomerulonephritis brought on by a vascular graft infection, with rapidly deteriorating kidney function despite extraction of the infected graft and 3 weeks of antibiotherapy with achievement of infection control. Kidney function improved after the introduction of corticosteroids. This case highlights the potential role of corticosteroids in selected cases of staphylococcus-induced glomerulonephritis, particularly those in which the infection is under control.

Superior semicircular canal dehiscence is caused by a bone defect on the roof of the superior semicircular canal. The estimated prevalence when unilateral varies between 0.4% and 0.7% and is still unknown when bilateral. Patients may present with audiologic and vestibular symptoms that may vary from asymptomatic to disabling. We report a case of a 72-year-old Caucasian woman presented to otolaryngology department reporting imbalance, bilateral pulsatile tinnitus, hypoacusis while being very sensitive to certain sounds. Physical examination was unremarkable, except for the Rinne test that was negative in both sides. The patient underwent an audiometry revealing a mild bilateral conductive hearing loss. A temporal bone CT scan was performed which evidenced bilateral superior semicircular canal dehiscence. Cervical vestibular evoked myogenic potentials and electrocochleography confirmed diagnosis. Although rare, superior semicircular canal dehiscence shall be considered in conductive hearing loss with vestibular symptoms.

Background Registries have a major role in patient follow-up, prospectively collecting real life data, they help us to better understand rare diseases like scleroderma. These atabases allow standardization of daily clinical practice, introducing decision support tools and improving quality and health outcomes. They also allow drug and device surveillance; scientific societies, academia and regulatory authorities can analyze their selected core set of data. Methods Our web-based registry - RIDAI (Informatic Registry of Autoimmune Diseases; www.ridai.org), was created in 2003 by the Portuguese Society Internal Medicine to support all Autoimmunity Clinics in Portugal; currently collecting data from 40 centres, with 4825 patients with different diseases. It has been constantly developped to comprise all the updated tools to measure important domains in autoimmune diseases. Demographic and clinical data were collected and analysed in Microsoft Excel 2013. Results Short presentation and demonstration of RIDAI, online since 2013. The scleroderma cohort has 326 patients, 227 limited and 99 diffuse subtypes, 43 (13.2%) males, mean age 56.4 (range 3–90), longest follow-up since 1993. Comorbidities are highly prevalent in our cohort: dyslipidemia in 46 patients, hypertension in 40, peripheral vascular disease in 22, osteoporosis in 20, heart failure in 13, diabetes in 10 and coronary heart disease in 3 patients. Most frequently used tools include Modified Rodnan Skin Score (mRSS), Visual Analogic Scale (VAS), Joint Count, EUSTAR Systemic Sclerosis Activity Scale (EUSTAR SSAS), the UCLA Scleroderma Clinical Trials Consortium Gastrointestinal Scale (SCTC GIT), Health Assessment Questionaire (HAQ) and Short Form-36 (SF-36). MRSS has a mean value of 9.1. Patient VAS has a mean 39.6; physician VAS mean 38.6. Painful Joint Count has a mean value of 3.0; tender Joint Count mean 1.6. The EUSTAR SSAS has a mean value of 1.27. SCTC GIT mean value is 0.52. HAQ mean value is 1.0; SF-36 mean is 103.9. Biological disease modifying drugs were prescribed off-label in 10 patients, for severe skin and joint disease. Conclusions The RIDAI is the largest portuguese scleroderma registry. It9s a versatile application and can be articulated with other platforms, with direct contribution to international multicentric studies. The presentation of the first 326 patients illustrates the complexity and heterogeneity of systemic sclerosis and of this cohort of patients. Treating to target and using standardized monitoring tools, Portuguese Internal Medicine physicians confirm good treatment performance, with a positive impact on indexes, specially mean mRSS and quality of life (HAQ, SF-36); enabling comparision with best practices. Disclosure of Interest None declared